Transcriptional activation of the cardiac homeobox gene CSX1/NKX2-5 in a B-cell chronic lymphoproliferative disorder

Xinying Su, Véronique Della-Valle, Eric Delabesse, Zahia Azgui, Roland Berger, Hélène Merle-Béral, Olivier A. Bernard, Florence Nguyen-Khac

Résultats de recherche: Contribution à un journalArticleRevue par des pairs

7 Citations (Scopus)

Résumé

Homeobox containing transcription factors are frequently deregulated in human hematologic malignant diseases either indirectly through an abnormality of an upstream factor, or directly through rearrangement of the gene itself. Study of T-cell acute lymphoblastic leukemia identified the related non-clustered homeobox transcription factors, TLX1 and TLX3, as frequently ectopically expressed as a result of chromosomal translocations. We report the deregulation of a non-clustered homeobox gene in a new type of t(5;14)(q35;qll) translocation in a mature peripheral B-cell leukemia. This translocation results in the ectopic expression of the CSX1/NKX2-5 gene on chromosome 5q35 due to its juxtaposition to the TCR δ gene on chromosome 14q11. Expression of the CSX1/NKX2-5 protein conferred enhanced replating potential to transduced murine bone marrow cells. Our study establishes that deregulation of homeobox encoding genes is not restricted to acute leukemic proliferations, but is also observed in chronic malignant diseases.

langue originaleAnglais
Pages (de - à)1081-1085
Nombre de pages5
journalHaematologica
Volume93
Numéro de publication7
Les DOIs
étatPublié - 1 juil. 2008
Modification externeOui

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